儿童和成人继发性aa -淀粉样变:分型、病因、发病机制、诊断和治疗

A. A. Stepanova, N. D. Savenkova
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引用次数: 0

摘要

本文综述了目前淀粉样变的命名和分类,儿童和成人继发性aa -淀粉样变的病因、发病机制、病程、诊断和治疗特点。在系统性淀粉样变性中,继发性aa -淀粉样变性占40 - 60%。本文报道了儿童MEFV基因纯合或杂合突变引起的家族性地中海热(周期性疾病)继发性aa -淀粉样变的发病特点。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Secondary AA-amyloidosis in pediatric and adult patients: classification, etiology, pathogenesis, diagnosis, treatment
The literature review summarizes information about the current nomenclature and classification of amyloidosis, the features of the etiology, pathogenesis, course, diagnosis and treatment of systemic secondary AA-amyloidosis in children and adult patients. Among the systemic forms of amyloidosis, secondary AA-amyloidosis accounts for 40–60 % of cases. The literature data on the features of the development of secondary AA-amyloidosis in familial Mediterranean fever (periodic illness) due to homozygous or heterozygous mutation of the MEFV gene in children are presented.
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